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Neonatal Acute Respiratory Distress due to SP-B Deficiency

Neonatal Acute Respiratory Distress due to Surfactant Protein B Deficiency


Presentation

  • Written for pulmonary clinicians and scientists alike, Molecular Basis of Lung Disease: Insights from Rare Disorders is a comprehensive and invaluable nesource that sheds new light on the molecular mechanisms influencing the clinical presentation and[books.google.de]
  • (OI) PowerPoint Presentation: A-aDO2 PAO2-Pao2 Normal range in a new born :5-15 Abnormal :15-40 Definitely abnormal: 40 PowerPoint Presentation: a/A Ratio Ratio of Pao2 to PAO2 It is a better indicator of gas exchange as the ratio is less affected by[authorstream.com]
  • We present a patient with a novel point mutation resulting in hereditary Surfactant Protein-B (SP-B) deficiency.[content.iospress.com]
  • SP-B mRNA was present in all lung samples except that of the case patient, in which no signal was detected even after prolonged exposure.[nejm.org]
  • Since surfactant normally develops late in prenatal life it usually is not present in the very premature infant of about 26-36 weeks of gestational age.[rarediseases.org]
Pulmonary Disorder
  • SP-B was readily detected in the lung tissue of the control patients with various pulmonary disorders but was absent in the case patient's lung tissue ( Figure 1 ). SP-A was readily detected in all lung samples in roughly comparable amounts.[nejm.org]
Pleural Effusion
  • […] fallowing features are essential 1)Tachypnea (RR 60 PER MIN..) 2)Retractions( intercoastal retractions and /or sub coastal) 3) Expiratory grunt PowerPoint Presentation: Transient tachypnea Pneumonia Aspiration syndromes Pneumothorax Pulmonary hypoplasia Pleural[authorstream.com]
Suggestibility
  • Each chapter focuses on the pathogenic mechanisms and therapeutic targets suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models[books.google.de]
  • Anne Murphy and Arnold Strauss for helpful suggestions.[nejm.org]
  • We suggest heightened vigilance and consultation with a reference laboratory in detecting similar mutations in term newborns presenting with unexplained respiratory failure.[content.iospress.com]
  • (PMID: 16456143) Hersh CP … Silverman EK (American journal of respiratory and critical care medicine 2006) 3 22 45 60 Family-based association tests suggest linkage between surfactant protein B (SP-B) (and flanking region) and respiratory distress syndrome[genecards.org]
  • PowerPoint Presentation: SCORE 0 1 2 Respiration (rate/min) 80 Cyanosis (Spo2 40% ambient oxygen Retractions None Mild Moderate-Severe Grunting None Audible with steth Audible without steth Air entry Good Decreased Barely audible Downe’s Score 7 is suggestive[authorstream.com]

Workup

Pleural Effusion
  • […] fallowing features are essential 1)Tachypnea (RR 60 PER MIN..) 2)Retractions( intercoastal retractions and /or sub coastal) 3) Expiratory grunt PowerPoint Presentation: Transient tachypnea Pneumonia Aspiration syndromes Pneumothorax Pulmonary hypoplasia Pleural[authorstream.com]

Treatment

  • […] suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models, clinical presentation, diagnostic approaches, conventional management and treatment[books.google.de]
  • Standard Therapies Treatment of infant respiratory distress syndrome consists of mechanical or physical breathing assistance such as positive end expiratory pressure (PEEP). Other treatment is symptomatic and supportive.[rarediseases.org]
  • , which became a more established treatment modality.[healio.com]
  • Intratracheal surfactant Supplementary oxygen as needed Mechanical ventilation as needed Specific treatment of RDS is intratracheal surfactant therapy.[msdmanuals.com]
  • A synthetic surfactant is commercially available for use in the treatment of respiratory distress syndrome of the newborn. See also acute respiratory distress syndrome. sur·fac·tant ( sŭr-fak'tănt ), 1.[medical-dictionary.thefreedictionary.com]

Prognosis

  • Prognosis with treatment is excellent; mortality is 10%. With adequate ventilatory support alone, surfactant production eventually begins, and once production begins, RDS resolves within 4 or 5 days.[msdmanuals.com]
  • Finally, the prognosis for infants with congenital alveolar proteinosis has been uniformly poor, even with the use of extracorporeal membrane oxygenation. Therapeutic whole-lung lavage has also been considered 4 .[nejm.org]

Etiology

  • Transient tachypnea Pneumonia Aspiration syndromes Pneumothorax Pulmonary hypoplasia Pleural effusions Airway problems Hyaline membrane disease Pneumonia Pulmonary hemorrhage Air leak syndromes Pulmonary hypoplasia Bronchopulmonary dysplasia Laryngomalacia Etiology[authorstream.com]
  • The etiology of BPD is problematic and may be due to oxygen, overventilation or underventilation.[en.wikipedia.org]

Epidemiology

  • Each chapter focuses on the pathogenic mechanisms and therapeutic targets suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models[books.google.de]
  • Relevant External Links for SFTPB Genetic Association Database (GAD) SFTPB Human Genome Epidemiology (HuGE) Navigator SFTPB Atlas of Genetics and Cytogenetics in Oncology and Haematology: SFTPB No data available for Genatlas for SFTPB Gene Polymorphic[genecards.org]
Sex distribution
Age distribution

Pathophysiology

  • HMD - Pathophysiology: HMD - Pathophysiology Terminal airspaces are not uniform throughout the lung There is a range of critical opening and critical closing pressures in various alveoli Lung Function in HMD: Lung Function in HMD Lung Compliance is also[authorstream.com]
  • This chapter describes the pathophysiology and molecular biology of hereditary SP-B deficiency.[ommbid.mhmedical.com]
  • Pathophysiology of respiratory distress syndrome. The above scenario is repeated nightly throughout the United States with private pediatricians caring for such infants on a regular basis.[healio.com]
  • Pathophysiology [ edit ] The lungs of infants with respiratory distress syndrome are developmentally deficient in a material called surfactant, which helps prevent collapse of the terminal air-spaces (the future site of alveolar development) throughout[en.wikipedia.org]

Prevention

  • Extensive testing of each preparation has demonstrated that both products are efficacious in preventing and treating RDS and preventing the complications of pulmonary airleak.[healio.com]
  • The only way to prevent the need for surfactant replacement therapy is to prevent a premature birth.[medical-dictionary.thefreedictionary.com]
  • Figure 5 The airspaces are lined with a lipoprotein complex called pulmonary surfactant, named for its ability to maintain normal respiratory mechanics by reducing surface tension at the air/liquid interface, preventing the lungs from collapsing at the[webs.ucm.es]
  • Prevention [ edit ] Giving the mother glucocorticoids speeds the production of surfactant. For very premature deliveries, a glucocorticoid is given without testing the fetal lung maturity.[en.wikipedia.org]
  • Secondly, ethical considerations prevented us from performing longitudinal measurements; accordingly, we could not assess the pattern of postnatal C-proSP-B development.[bmcpediatr.biomedcentral.com]

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